Rosai-Dorfman disease mimicking craniopharyngioma: a challenging differential diagnosis. Illustrative case

Rosai-Dorfman disease (RDD) is a rare non-Langerhans histiocytic disorder with less than 5% central nervous system involvement and is often mistaken for meningioma given the similarity in imaging features. The authors present the unique case of a 44-year-old female who presented with ongoing visual impairment.

A purely suprasellar mass was noted on magnetic resonance imaging and was initially diagnosed as craniopharyngioma. Unexpectedly, the pathology report revealed RDD.

To date, only six cases of sellar RDD have been reported, and our case is the first reported with a purely suprasellar presentation. No standard treatment has been established for RDD, and next-generation sequencing may be a promising therapeutic option.